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More recently, his father reports he has not worn his
implant at all over the last few months. Of note, family
dynamics seem to play a role in this patient’s device use.
The third pediatric patient was 3 years old at the time
of implantation. As of the 3-month postoperative evalu-
ation, PBK-word scores were 96%with the nonimplanted
ear alone and 32% with the implant alone. In the bimodal
condition the word score was 96% attributable to the
ceiling effect from having one normal hearing ear.
Importantly, the combined signal did not cause a decre-
ment in performance. On the sentence test, with noise-
front she scored 100% in the nonimplanted ear, 70% with
the CI alone, and 100% in the bimodal condition. Her
father reports that the patient no longer asks where sound
is coming from and responds better to sound in general.
Overall, the children demonstrated varying degrees of
open-set speech perception in the implanted ear and bilat-
eral improvement in the presence of background noise.
However, these few children introduce some of the issues
related to expectations after a prolonged duration of deaf-
ness and the impact of device use on performance.
Most recently, a family presented with their 6-month
old who was diagnosed with sensorineural hearing loss.
The family had done extensive research and asked many
appropriate questions. At their request, the child under-
went a cochlear implant evaluation at our center. After
extensive counseling, the family elected to proceed with
cochlear implantation, at the age of 11 months. There are
not yet any postoperative data available.
DISCUSSION
Perhaps the least understood aspect of unilateral hear-
ing loss is determining if and when treatment is indicated.
Some patients have to the ability to adapt well without
any intervention. Although adults who have experienced
postlingual SSD can endorse certain deficits or listening
difficulties, the same cannot be assumed of children.
Experience suggests that some children benefit from
noninvasive interventions; however,
determining
optimal treatment and timing for a given patient remains
a challenge. Some children and adults also overcome
such deficits without intervention.
The other available treatment options for SSD do not
restore hearing to the affected ear and hence, lack the
advantages of binaural hearing that require sound to
arrive at each ear independently for the processing of
timing and pitch differences to be integrated by the brain.
It should be noted that both the CROS and bone-anchored
hearing aids may have undesirable effects in certain
listening situations including hearing in noise, especially
when noise is present on the side with the implant and
may be routed to the better hearing ear, worsening the
signal-to-noise ratio and making listening more difficult.
Cochlear implants may overcome these issues, but should
not be expected to restore all of the benefits of
binaural hearing.
Studies such as our own on SSD CI are hampered by
our inability to fully measure the efficacy of the
treatment. Subjective improvement of localization and
speech understanding in difficult listening situations in
real-life situations with the addition of the second ear
after implantation may actually be more important than
our ability to quantify this with currently available tools
and methods. It is possible that the tests currently being
used are not sensitive enough to accurately reflect sub-
jective patient reports until a certain level of competence
is reached. As we move forward with evaluating SSD
candidacy for cochlear implantation, it will be important
to devise measurement tools that can better reflect the
binaural advantage in the sound field in the presence of a
normal or near-normal ear. Next we consider the factors
that we use to consider candidacy for SSD CI on the basis
of our experience so far.
Candidacy Considerations
As observed in Table 1, our patients were diverse in
their baseline characteristics including both demo-
graphics and audiometric characteristics. In some
patients, the better hearing ear was in the normal range,
but threatened in some way as in the case of an inner ear
malformation predisposing to progressive hearing loss or
as yet minimally symptomatic retrocochlear pathology in
an only-hearing ear. Patients differed significantly in
their motivating factors for pursuing cochlear implan-
tation be it tinnitus suppression, trouble in difficult
listening situations, or anticipated hearing loss in an
only-hearing ear. Many more patients with SSD have
been evaluated for cochlear implantation at our center
and this experience has allowed us to define the following
parameters for SSD CI candidacy.
Absolute Indication: Late Stage Unilateral Me´nie`re’s
Disease
Patients with late stage Me´nie`re’s disease may struggle
with intractable vertigo from an ear essentially nonfunc-
tional from an auditory perspective. With a simultaneous
labyrnthectomy and ipsilateral cochlear implant, patients
can have definitive treatment of their vertigo while
bringing their ‘‘ear back to life’’ all during an outpatient
ambulatory procedure. In 2013, Hansen et al. reported on
the results of cochlear implantation in patients with
Me´nie`re’s disease who progressed to profound sensor-
ineural hearing loss with one ear. They reported signifi-
cant improvement in word and sentence scores, though
ability to localize sound in this cohort showed much more
modest improvement (13). We have had similar experi-
ence with our cohort and we think this provides a hopeful
option for patients who have often had years of suffering
with their disease to both alleviate their vertigo and
rehabilitate their hearing.
Absolute Indication: An ‘‘At Risk’’ Only Hearing Ear
Though rare, a threatened only hearing ear, for
example, an acoustic neuroma or other retrocochlear
pathology, is an important consideration for a cochlear
implant. These patients live in fear of the possibility of 1
day waking up suddenly deaf ill equipped to handle the
D. R. FRIEDMANN ET AL.
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